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The so-called “one-and-a-half” ventricular repair: where are we after 40 years?
- Ujjwal Kumar Chowdhury, Robert H. Anderson, Niraj Nirmal Pandey, Sundeep Mishra, Lakshmi Kumari Sankhyan, Niwin George, Maroof A. Khan, Shikha Goja
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- Journal:
- Cardiology in the Young / Volume 33 / Issue 9 / September 2023
- Published online by Cambridge University Press:
- 03 July 2023, pp. 1497-1505
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Objectives:
The indications, timing, and results of the so-called “one-and-a-half ventricle repair”, as a surgical alternative to the creation of the Fontan circulation, or high-risk biventricular repair, currently remain nebulous. We aimed to clarify these issues.
Methods:We reviewed a total of 201 investigations, assessing selection of candidates, the need for atrial septal fenestration, the fate of an unligated azygos vein and free pulmonary regurgitation, the concerns regarding reverse pulsatile flow in the superior caval vein, the growth potential and function of the subpulmonary ventricle, and the role of the superior cavopulmonary connections as an interstage procedure prior to biventricular repair, or as a salvage procedure. We also assessed subsequent eligibility for conversion to biventricular repair and long-term functional results.
Results:Reported operative mortalities ranged from 3% to 20%, depending on the era of surgical repair with 7% risk of complications due to a pulsatile superior caval vein, up to one-third incidence of supraventricular arrhythmias, and a small risk of anastomotic takedown of the superior cavopulmonary connection. Actuarial survival was between 80% and 90% at 10 years, with two-thirds of patients in good shape after 20 years. We found no reported instances of plastic bronchitis, protein-losing enteropathy, or hepatic cirrhosis.
Conclusions:The so-called “one-and-a-half ventricular repair”, which is better described as production of one-and-a-half circulations can be performed as a definitive palliative procedure with an acceptable risk similar to that of conversion to the Fontan circulation. The operation reduces the surgical risk for biventricular repair and reverses the Fontan paradox.
Fontan failure: phenotypes, evaluation, management, and future directions
- Ujjwal Kumar Chowdhury, Niwin George, Lakshmi Kumari Sankhyan, Doniparthi Pradeep, Chaitanya Chittimuri, Abhinavsingh Chauhan, Niraj Nirmal Pandey, Shikha Goja
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- Journal:
- Cardiology in the Young / Volume 32 / Issue 10 / October 2022
- Published online by Cambridge University Press:
- 22 June 2022, pp. 1554-1563
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Objectives:
Management of “failing” and “failed” Fontan circulation, particularly the indications, timing, and type of re-intervention, currently remains nebulous. Factors contributing to pathogenesis and mortality following Fontan procedure differ between children and adults.
Methods:Since organ systems in individual patients are affected differently, we searched the extant literature for a “failing” and “failed” Fontan reviewing the clinical phenotypes, diagnostic modalities, pharmacological, non-pharmacological, and surgical techniques employed, and their outcomes.
Results:A total of 410 investigations were synthesised. Although proper candidate selection, thoughtful technical modifications, timely deployment of mechanical support devices, tissue-engineered conduits, and Fontan takedown have decreased the peri-operative mortality from 9 to 15% and 1 to 3% per cent in recent series, pernicious changes in organ function are causing long-term patient attrition. In the setting of a failed Fontan circulation, literature documents three surgical options: Fontan revision, Fontan conversion, or cardiac transplantation. The reported morbidity of 25% and mortality of 8–10% among Fontan conversion continue to improve in select institutions. While operative mortality following cardiac transplantation for Fontan failure is 30% higher than for other CHDs, there is no difference in long-term survival with actuarial 10-year survival of around 54%. Mechanical circulatory assistance, stem cells, and tissue-engineered Fontan conduit for destination therapy or as a bridge to transplantation are in infancy for failing Fontan circulation.
Conclusions:An individualised management strategy according to clinical phenotypes may delay the organ damage in patients with a failing Fontan circulation. At present, cardiac transplantation remains the last stage of palliation with gradually improving outcomes.
Criterions for selection of patients for, and results of, a new technique for construction of the modified Blalock-Taussig shunt
- Ujjwal K. Chowdhury, Panangipalli Venugopal, Shyam S. Kothari, Anita Saxena, Sachin Talwar, Ganapathy Subramaniam, Rajvir Singh, Kizakke K. Pradeep, Siddhartha Sathia, A. Sampath Kumar
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- Journal:
- Cardiology in the Young / Volume 16 / Issue 5 / October 2006
- Published online by Cambridge University Press:
- 20 September 2006, pp. 463-473
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Background: We describe alternative surgical techniques for construction of systemic-to-pulmonary arterial shunts, and propose criterions for their application in selected patients. Patients and methods: We constructed a variety of modified systemic-to-pulmonary arterial shunts, using polytetrafluroethylene grafts, in 92 selected patients with cyanotic congenital heart disease and anomalies of the aortic arch and systemic veins. Their age ranged from 7 days to 3.6 years, with a mean of 7.08 months. We performed 88 operations through a thoracotomy. Of this cohort, 60 patients underwent a second-stage operation, with 15 receiving a superior cavopulmonary connection, 16 a total cavopulmonary connection, and 29 proceeding to biventricular repair after a mean interval of 15.6 months. We have 21 patients awaiting their second or final stage of palliation. Results: There were five early (5.4%) and six late deaths (6.8%), two of which were related to construction of the shunts. At a mean follow-up of 45.29 months, the increase in diameter of pulmonary trunk and its right and left branches was uniform and significant (p value less than 0.001). Pulmonary arterial distortion requiring correction at the time of second-stage operation was observed in 5 patients (6.1%). Adequate overall palliation was achieved in 98% of the cohort at 8 months, 91% at 12 months, and 58% at 18 months. Conclusions: Patients with a right- or left-sided aortic arch and right-sided descending thoracic aorta, those with anomalies of systemic venous drainage masking the origin of great arterial branches, and those withdisproportionately small subclavian arteries, constitute the ideal candidates for our suggested modification of the construction of a modified Blalock-Taussig shunt. The palliation provided by these shunts was satisfactory, with predictable growth of pulmonary arteries, insignificant distortion in the great majority, and easy take-down.